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CASE REPORT
Year : 2022 | Volume
: 26
| Issue : 3 | Page : 15-18
Eccrine porocarcinoma at unusual location
Suyash S Tomar1, Bhagyashree B Supekar1, Ravi Bhushan1, Jayesh I Mukhi1, Rajesh P Singh1, Dharitri Bhat2, Abhisek Jaiswal3
1 Department of Dermatology, Venereology and Leprology, Government Medical College, Nagpur, Maharashtra, India
2 Department of Pathology, Government Medical College, Nagpur, Maharashtra, India
3 Department of Radiology, Government Medical College, Nagpur, Maharashtra, India
Correspondence Address:
Dr. Bhagyashree B Supekar
Department of Dermatology, Venereology and Leprology, Government Medical College and Hospital, Nagpur - 440 003, Maharashtra
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/jdds.jdds_40_21
Porocarcinoma is a malignant tumor arising from intraepidermal eccrine duct epithelium or acrosyringium with an aggressive course. Sites of predilection include lower extremities (50%), trunk (24%), and head and neck (24%), while it is uncommon in the upper extremities and genitals. We report a case of eccrine porocarcinoma in the axillary region with axillary lymph node involvement and pulmonary metastasis in a 78-year-old male.
Keywords: Axilla, immunohistochemistry, porocarcinoma, pulmonary metastasis
Eccrine porocarcinoma is malignant sweat gland tumors representing 0.005%–0.01% of all cutaneous neoplasm.[1] This tumor originates from the intraepithelial parts of the eccrine sweat glands and follows an aggressive course. The commonest site of occurrence are the lower extremeties.[2] Propensity to form multiple cutaneous metastases is an unusual feature of eccrine porocarcinoma.[3] Approximately, 250 cases have been reported in literature till now, of which only two cases were documented in the axilla.[4]
Case ReportA 78-year-old male patient presented with single dark colored raised lesion over the left axilla of 8 months duration with swelling over the entire left upper limb for 3 months. There was no previous history of similar lesions, radiation injury, prolonged sun exposure or immunosuppression, chronic smoking, or alcoholism. He had no history of cough, hemoptysis, and exertional dyspnea. There was no history of any systemic disease. There was no history of breathlessness, headache, or convulsion in the past 8 months.
On cutaneous examination, a single, flesh colored, firm to hard lobulated growth of size 4 cm × 3 cm was present over the left axilla [Figure 1]a. Rest cutaneous examination was normal. A detailed general examination revealed diffuse pitting edema over the entire left upper limb extending from the dorsum of the hand up to the middle of the left arm.[Figure 1]b Multiple, nontender enlarged lymph nodes of sizes varying from 3 cm × 2 cm to 1 cm × 1 cm, were palpable in the left axilla. Both the breasts were normal. Rest general examination was normal. On the chest ausculatation, air entry was bilaterally equal and no adventitious sound was heard. Systemic examination revealed no abnormality.
Figure 1: (a and b) Single, flesh colored, firm to hard lobulated growth over left axilla. (a) diffuse pitting edema over entire left upper limb extending from the dorsum of the hand up to the middle of the left arm (b)Routine hematological investigations were within the normal limits. Serology for human immunodeficiency virus (HIV) and hepatitis B virus was nonreactive. Local ultrasound of the lesion revealed an ill-defined heterogeneous hyperechoic solid cystic lesion of size 1.6 cm × 2 cm × 1.7 cm arising from the left axilla suggestive of malignant etiology. There were few enlarged lymph nodes in the left axilla with the largest being 2.9 cm × 1.4 cm. Chest X-ray revealed bilateral hilar prominence. Fine-needle aspiration cytology (FNAC) of the nodule from axilla revealed clusters of malignant cells [Figure 2]a with dense granular eosinophilic cytoplasm with squamous differentiation [Figure 2]b, nuclear pleomorphism and prominent nucleoli [Figure 2]c, few columnar cells, and desmosomes [Figure 2]d.
Figure 2: (a-d) Fine-needle aspiration cytology of the nodule from axilla showing clusters of malignant cells (a) (H and E, ×40); with dense granular eosinophilic cytoplasm with squamous differentiation (b) (Pap, ×40); nuclear pleomorphism and prominent nucleoli (c) (Pap, ×100); columnar cells and desmosomes (d) (Pap, ×40)Computerized tomography of thorax plain and contrast revealed mildly heterogeneously enhancing lobulated lesion in the left axillary region, suggestive of malignant neoplastic etiology [Figure 3]a, metastatic left axillary lymphadenopathy [Figure 3]b, and few intraparenchymal, fissural, subpleural metastatic nodules [Figure 3]c with metastatic hilar, and mediastinal lymphadenopathy [Figure 3]d.
Figure 3: (a-d) Computed tomography of the thorax plain and contrast revealed mildly heterogeneously enhancing lobulated lesion in the left axillary region, suggestive of malignant etiology (a), metastatic left axillary lymphadenopathy (b), and few intraparenchymal, fissural, and subpleural metastatic nodules (c) with metastatic hilar and mediastinal lymphadenopathy (d)A local excisional biopsy with wide margin of 2 cm was performed with lymph node dissection was performed and sent for histopathological examination [Figure 4]. Histopathology from nodular lesion revealed nests, sheets, and lobules of malignant cells surrounded by fibrocollagenous septae [Figure 5]a, round-to-oval tumor cell (poroma cells) with vacuolated eosinophilic cytoplasm [Figure 5]b, nuclear pleomorphism, mitotic figures, prominent nucleoli [Figure 5]c and formation of desmosomes [Figure 5]d, and suggestive of eccrine porocarcinoma. Periodic acid Schiff staining was negative [Figure 5]e and [Figure 5]f. Immunohistochemistry staining of the nodular lesion revealed Cytokeratin (CK7) positivity of tumor cells [Figure 6]a and epidermal membrane antigen (EMA) positivity of tumor cells [Figure 6]b. Based on clinical features, radiological investigations, cyto-histopathology, and immunohistochemistry staining a diagnosis of axillary eccrine porocarcinoma with local lymph node and pulmonary metastasis were reached. The patient was referred to the oncology and radiation department for further management and he was started on injection docetaxel 80 mg and injection cisplatin 50 mg. However, the patient was lost to further follow-up.
Figure 4: A wide margin local excisional biopsy with lymph node dissection was performed
Figure 5: (a-f) Histopathology from nodular lesion revealed nests, sheets, and lobules of malignant cells surrounded by fibrocollagenous septae (a) (H and E, ×4); clusters of round-to-oval tumor cells (poroma cells) with vacuolated eosinophilic cytoplasm (b) (H and E, ×10); nuclear pleomorphism, mitotic figures, and prominent nucleoli (c) (H and E, ×40); desmosomes formation (d) (H and E, ×40); Periodic acid schiff stain of nodular lesion (e: ×4, f: ×40), suggestive of eccrineporocarcinoma.
Figure 6: (a and b) Immunohistochemistry of nodular lesion revealed CK7 positivity of tumor cells (a: ×40); epidermal membrane antigen positivity of tumor cells (b: ×40) Discussion Pinkus and Mehregan in 1963, first described an adnexal tumor with metastatic potential as “epidermotropiceccrine carcinoma,”[5] However, the term “eccrineporocarcinoma” was introduced by Mishisma and Moriko in 1969.[6] It is a malignant tumor arising from intraepidermal eccrine duct epithelium or acrosyringium with an aggressive course. It may develop either de novo or from a pre-existing benign eccrineporomaor hydroacanthoma simplex. It is seen in the sixth to eighth decades of life with equal sex incidence. The tumor may be associated with immunocompromised states such as HIV infection, diabetes, sarcoidosis, and organ transplantation.[7]
Eccrineporocarcinoma usually presents as a single ulcerated nodule or plaque, or as a polypoid or verrucous lesion. Sites of predilection include lower extremities (50%), trunk (24%), and head and neck (24%), while it is uncommon in the upper extremities and genitals.[8] Our patient had the involvement of axilla which is a very rare site. Cytology plays an important role in the diagnosis of primary cutaneous malignant epithelial tumors. Histologically most of these tumors exhibit acanthotic epidermis with tumor cells invading into the dermis forming nests, sheets, and cords with a focal insular pattern. The tumor cells are polygonal or fusiform having round-to-oval pleomorphic nuclei with indistinct nuclear borders and a variable amount of cytoplasm. Immunohistochemical staining with EMA, cytokeratin7, and carcinoembryonic antigen is found to be strongly positive.[9] In our case, differential diagnoses of metastatic pulmonary adenocarcinoma, cutaneous squamous cell carcinoma (SCC), and occult breast carcinoma can be considered. Pulmonary adenocarcinoma can be differentiated from eccrine porocarcinoma by pulmonary signs/symptoms and positive IHC staining for CK5/6, CK7, TTF1, Napsin A, and P63.[10] In our case, no lump in the breast was palpable and local sonography and mammography were normal. CK 5 and CK 17 positivity were reported in 16% of occult/metastatic breast carcinoma.[11] Porocarcinoma can mimic SCC if it shows pagetoid intraepidermal spread, colonizes the adnexal structure, acantholysis, and pseudogland formation. Ductal differentiation and the presence of intracytoplasmic lumina helps in its distinction from cutaneous SCC.[12] Treatment modality for eccrine porocarcinoma is wide local excision with broad tumor margins and regional lymph node dissection or Moh's micrographic surgery. It can be combined with postoperative radiotherapy. The recurrence rate in eccrine porocarcinoma is 20% and metastasis to lymph nodes accounts for 20% approximately. Systemic metastases are known to have occurred in multiple cases associated with mortality rate up 75%–80%.[13] We report a case of axillary porocarcinoma with local lymph node metastasis and lymphedema. There are two reports of axillary porocarcinoma in the literature described by Devi et al.[7] and Arslan et al.[13][Table 1]. Devi et al. reported axillary porocarcinoma with local lymph node metastasis. Arslan et al. described axillary porocarcimoma with pulmonary and lymph node metastasis. To the best of our knowledge, this is the first case report of axillary porocarcinoma in an immunocompetent patient with pulmonary and local lymph node metastasis, from India.
In conclusion, eccrineporocarcinoma is very rare in occurrence and has a nonspecific presentation. Thus, the condition might be misdiagnosed as SCC, basal cell carcinoma, seborrheic keratosis, or metastatic adenocarcinoma which has a similar presentation. Delay in diagnosis may be associated with increased mortality, as the tumor carries a malignant potential. Therefore, knowledge about this entity is necessary among clinicians for early accurate diagnosis and appropriate treatment.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
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Conflicts of interest
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